C1 Inhibitor
Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema
... reduced C1 inhibitor synthesis in patients with type 1 HANE consistent with a single functional C1 inhibitor ...of C1 inhibitor in HANE type 1 appears to result from (a) the ...
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Ecallantide is a novel treatment for attacks of hereditary angioedema due to C1 inhibitor deficiency
... the C1 inhibitor protein is a rare disease, characterized by paroxysms of edema formation in the subcutis and in the ...specific inhibitor of plasma kallikrein is an innovative medicinal ...
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Review of the Manitoba cohort of patients with hereditary angioedema with normal C1 inhibitor
... with C1 INH or tranexamic acid for acute treatment of attacks (4 with C1 INH and 1 with tranexamic ...(C1 inhibitor and tranexamic acid) were ...
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Glucocorticoid receptor gene polymorphisms in hereditary angioedema with C1-inhibitor deficiency
... by C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant ...disorder. C1-INH-HAE is characterized by edema – formation, which may occur in response to ...of C1-INH-HAE ...
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Transinhibition of C1 inhibitor synthesis in type I hereditary angioneurotic edema
... of C1 inhibitor (C1 INH) in certain patients with the autosomal dominant disorder hereditary angioneurotic edema, we studied expression of C1 INH in fibroblasts in which the mutant and wild ...
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A nationwide survey of hereditary angioedema due to C1 inhibitor deficiency in Italy
... to C1 Inhibitor (C1-INH) deficiency (C1-INH-HAE) is a rare auto- somal dominant disease due to reduced C1-INH plasma levels (C1-INH-HAE type I) or to the presence of a ...
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Proteolytic inactivation of plasma C1 inhibitor in sepsis
... Because C1 inhibitor (C1-Inh) regulates the activation of both cascade systems, we studied the characteristics of plasma C1-Inh in 48 patients with severe sepsis on admission to the Intensive ...
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Platelet C1 inhibitor A secreted alpha granule protein
... contain C1- inhibitor. By means of monospecific antibody to C1- inhibitor, a competitive enzyme-linked immunosorbent assay (CELISA) was developed to measure directly platelet C1- ...of ...
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Demonstration of modified inactive first component of complement (C1) inhibitor in the plasmas of C1 inhibitor deficient patients
... (C1) inhibitor plays a critical role in the regulation of the classical complement pathway and the contact system, and the deficiency of C1 inhibitor protein or function is associated with ...
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Nine year follow-up of a rare case of angioedema due to acquired C1-inhibitor deficiency with late onset and good response to attenuated androgen
... when C1 inhibitor esterase (C1-INH) deficiency was ...negative. C1-INH and complement fraction C4 plasma levels were significantly decreased at all measurements, but no diagnostic criteria for ...
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Abstracts from the 10th C1-inhibitor deficiency workshop
... a C1-inhibitor workshop in ...low C1 inhibitor ...Recombinant C1-inhibitor and a C1-inhibitor concentrate were offered in ED in a small amount from 2015 and it was ...
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Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor
... of C1 esterase inhibitor (C1-INH) caused by mutations of the C1-INH ...normal C1-INH concentration and activity with respect to C1 esterase inhibition, ruling out both types of ...
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Pediatric hereditary angioedema due to C1-inhibitor deficiency
... the C1 inhibitor (C1-INH) is a rare, life-threatening ...and C1-INH replacement ...option. C1-INH replacement therapy is also an effective and safe agent for ...
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Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency
... This consensus recommendation was conceptualized at a meeting in May 2014 of the Hereditary Angioedema Medical Board and was further discussed at a meeting in October 2015. A series of conference calls allowed further ...
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Plasma levels of C1 inhibitor complexes and cleaved C1 inhibitor in patients with hereditary angioneurotic edema
... C1- inhibitor (C1(-)-Inh) catabolism in plasma of patients with hereditary angioneurotic edema (HANE) was assessed by measuring the complexes formed by C1(-)-Inh with its target proteases ...
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Serial change of C1 inhibitor in patients with sepsis: a prospective observational study
... and C1-INH activity The patients’ clinical background and course including age, sex, Acute Physiological and Chronic Health Evalu- ation (APACHE) II score, Sequential Organ Failure Assessment (SOFA) score, ...
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Administration of C1 Inhibitor Reduces Neutrophil Activation in Patients with Sepsis
... at days 1 (median [range], 262 [36 to 1,435] pg/ml) and 3 (146.5 [22 to 1,199] pg/ml) in those receiving the placebo than in those receiving C1 inhibitor (76 [36 to 44,344] and 52.5 [30 to 152] pg/ml, ...
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Abdominal attacks and treatment in hereditary angioedema with C1-inhibitor deficiency
... in C1- inhibitor (HAE-C1INH), although HAE with normal C1-INH (HAEnC1) has been described ...[6]. C1-INH is a serine-protease inhibitor that regulates the activation of the coagulation, ...
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Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate
... to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is a serious condition that may result in life-threatening asphyxiation due to laryngeal ...plasma-derived C1-INH concentrate (pdC1-INH) ...
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The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency
... 3. Agostoni A, Aygören-Pürsün E, Binkley KE, Blanch A, Bork K, Bouillet L, Bucher C, Castaldo AJ, Cicardi M, Davis AE, De Carolis C, Drouet C, Duponchel C, Farkas H, Fáy K, Fekete B, Fischer B, Fontana L, Füst G, ...
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